Current status of immunogenetic studies on primary biliary cholangitis / 临床肝胆病杂志

ABSTRACT

Primary biliary cholangitis (PBC) has the pathological feature of progressive intrahepatic cholestasis caused by immune-mediated apoptotic necrosis of small biliary epithelial cells, with a risk of progression to bile duct fibrosis, liver cirrhosis, and hepatocellular carcinoma. PBC has immunogenetic characteristics, and the abnormal genetic regulation of immune response in patients with PBC includes abnormal immune response of T and B lymphocytes involving human leukocyte antigen (HLA) and non-HLA genes and taking pyruvate dehydrogenase complex-E2 in mitochondria of intrahepatic small biliary epithelial cells as the antigen. More than 30% of PBC patients have poor response to ursodeoxycholic acid treatment, and therefore, clarifying the mechanism of abnormal immune regulation in PBC has great clinical significance in guiding the immunotherapy for PBC.